Primary antiphospholipid syndrome: a low-grade auto-inflammatory disease?

Area of Science:

• Immunology
• Rheumatology
• Hematology

Background:

• Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by recurrent thrombosis and pregnancy morbidity.
• The role of inflammation and immune activation in primary APS (PAPS) pathogenesis remains incompletely understood.

Purpose of the Study:

• To investigate inflammation and immune activation markers in patients with thrombotic primary APS (PAPS).
• To identify clinical and laboratory factors associated with inflammation and immune activation in PAPS.

Main Methods:

• Compared PAPS patients (n=41) with inherited thrombophilia (IT, n=44) and controls (CTR, n=39).
• Measured inflammatory markers (hs-CRP, SAA, CRP-oxLDL-β2GPI) and immune activation markers (neopterin, sCD14) using ELISA.
• Employed regression models to analyze relationships between markers, clinical features, and antiphospholipid antibodies (aPLs).

Main Results:

• PAPS patients exhibited significantly higher levels of hs-CRP, SAA, CRP-oxLDL-β2GPI, neopterin, and sCD14 compared to IT and CTR groups.
• In PAPS, serum amyloid A (SAA) correlated with thrombosis number, neopterin with thrombosis type and number, and soluble CD14 (sCD14) with IgG β2GPI, age, and arterial thrombosis.
• CRP-oxLDL-β2GPI independently predicted SAA, and sCD14 and neopterin showed a mutual relationship.

Conclusions:

• Thrombotic PAPS is characterized by low-grade systemic inflammation and immune activation.
• These inflammatory and immune activation processes are associated with specific clinical manifestations and antiphospholipid antibody levels in PAPS.